1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-9-18-a 10.15844/pedneurbriefs-9-3-2 Seizure Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Vigabatrin Monotherapy for Infantile Spasms http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 03 1995 01 06 2016 9 3 18 18 Copyright: © 1995 The Author(s) 1995 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. ‘A simple, effective and well-tolerated treatment regime for West Syndrome’

The successful management of 21 children with infantile spasms and hypsarrhythmia using vigabatrin monotherapy is reported from the Alder Hey Children’s Hospital, Liverpool, UK.

 Infantile Spasms Vigabatrin Monotherapy Symptomatic Cause

The successful management of 21 children with infantile spasms and hypsarrhythmia using vigabatrin monotherapy is reported from the Alder Hey Children’s Hospital, Liverpool, UK. Age at onset of spasms was 3 to 16 months. A symptomatic cause was identified in 17(81%). Spasms were completely controlled in 17(81%) with an initial dose of vigabatrin 25-50 mg/kg/day, increasing to a maximum of 80-120 mg/kg/d in 3-5 days. At a mean 2 year follow-up, 14(67%) remained seizure-free, and vigabatrin was withdrawn in 4 without relapse. Only one patient failed to respond; this child had meningitis at 4 months and spasms were refractory to all AEDs, including ACTH. Transient drowsiness in 2 patients was the only side-effect noted. [1]

COMMENT. Vigabatrin has replaced ACTH and prednisone as the first-line treatment for West syndrome in the Liverpool Children’s Hospital. Dr Verity and colleagues in the UK have been very successful in their organization of a multicenter, comparative trial of sodium valproate and carbamazepine. A similar controlled trial of vigabatrin and ACTH based in Liverpool might be necessary to convince other centers to initiate a change in treatment of infantile spasms.

Of interest, only 4 children (10%) were seizure free following vigabatrin monotherapy for intractable epilepsy in a previous report from the Royal Liverpool Children’s Hospital. Complex partial seizures responded partially but myoclonic seizures were not benefited.(Gibbs et al. 1992; see Progress in Pediatric Neurology II, 1994, pp 104-5).

An open, add-on trial of vigabatrin in 20 children with Lennox-Gastaut syndrome, reported from Wien, Austria, showed 85% with a 50-100% reduction in seizure frequency, even after valproate dosage was reduced. Dyskinesia in 1 child was the only side-effect [2]. Serious mood disorders, depression and/or aggression, were the main reason for withdrawing vigabatrin in 9 (12%) of 73 adults with refractory epilepsy treated at the Meer & Bosch Epilepsy Centre, Heemstede, The Netherlands [3]. Vigabatrin results in a significant increase in brain GABA concentration by inhibiting GABA transaminase.

 Appleton RE ‘A simple, effective and well-tolerated treatment regime for West Syndrome’ Dev Med Child Neurol 1995 Feb 37 2 185 6 7851675 Feucht M Brantner-Inthaler S Gamma-vinyl-GABA (vigabatrin) in the therapy of Lennox-Gastaut syndrome: an open study Epilepsia 1994 Sep-Oct 35 5 993 8 7925171 10.1111/j.1528-1157.1994.tb02544.x Aldenkamp AP Vermeulen J Mulder OG Overweg J Van Parys JA Beun AM Gamma-vinyl GABA (vigabatrin) and mood disturbances Epilepsia 1994 Sep-Oct 35 5 999 1004 7925172 10.1111/j.1528-1157.1994.tb02545.x